CD7+, CD4-, CD8- acute leukemia: a syndrome of malignant pluripotent lymphohematopoietic cells.
CD7+, CD4-, CD8- acute leukemia: a syndrome of malignant pluripotent lymphohematopoietic cells.
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DOI：
10.1182/blood.v73.2.381.bloodjournal732381
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发表时间：
1989-02
期刊：
影响因子：
20.3
通讯作者：
J. Kurtzberg;T. Waldmann;M. Davey;S. Bigner;J. Moore;M. Hershfield;B. Haynes
中科院分区：
文献类型：
--
作者：
J. Kurtzberg;T. Waldmann;M. Davey;S. Bigner;J. Moore;M. Hershfield;B. Haynes
在我们对CD7+、CD4-、CD8-急性淋巴细胞白血病(ALL)细胞从淋巴谱系向骨髓谱系的体内转化进行初步观察之后(Proc Natl Acad Sci (USA) 81:253, 1984)，我们研究了另外八个具有这种白血病细胞表型的ALL病例。 CD7+、CD4-、CD8- 表型与独特的临床实体相关，这些患者主要为男性（小于 35 岁或大于 65 岁），常见纵隔和/或胸腺肿块、皮肤和中枢神经系统疾病、外周血白细胞计数高、骨髓原始细胞形态为 L1 或不归因于特定谱系。这些患者对急性淋巴细胞白血病或粒细胞白血病的常规化疗方案没有反应。无法定义常见的核型或 T 细胞基因重排模式。重要的是，所研究的八个患者的白血病细胞中有七个能够在体外进行多谱系（骨髓细胞、红细胞、单核细胞、巨核细胞和淋巴细胞）分化。数据表明，CD7+、CD4-、CD8- 白血病在许多情况下是未成熟造血细胞的白血病。开发针对这种白血病的新治疗方法对于改变其不良预后是必要的。
Following our initial observation of in vivo conversion of CD7+, CD4-, CD8- acute lymphoblastic leukemia (ALL) cells from lymphoid to myeloid lineages (Proc Natl Acad Sci (USA) 81:253, 1984) we have studied eight additional cases of ALL with this leukemic cell phenotype. The CD7+, CD4-, CD8- phenotype was associated with a distinct clinical entity with those affected predominantly male (either less than 35 years or greater than 65 years of age), with frequent mediastinal and/or thymic masses, skin and CNS disease, high peripheral WBC counts, and bone marrow blasts that were morphologically L1 or not ascribable to a specific lineage. These patients did not respond to conventional chemotherapeutic regimens for either acute lymphoid or myeloid leukemias. No common karyotype or T-cell gene rearrangement pattern could be defined. Importantly, seven of eight patient's leukemic cells studied were capable of multilineage (myeloid, erythroid, monocytoid, megakaryocytoid, and lymphoid) differentiation in vitro. Data is presented suggesting that CD7+, CD4-, CD8- leukemias, in many instances, are leukemias of immature hematopoietic cells. The development of novel therapeutic approaches to this form of leukemia will be necessary to alter its poor prognosis.